Hydrocysticercosis of the neck, also known as congenital cystic hydatidiform tumor of the neck or cystic lymphadenoma, is a congenital condition that originates from lymphatic tissue. It can occur in various parts of the body and is mostly a soft, fluctuating, painless mass that is not easily compressed and has good light transmission. Intracapsular injection of drugs is effective, safe and convenient, and is the treatment of choice. It is also a common disease in newborns as well as infants. It occurs around the neonatal period and is overlooked because of its small size and lack of obvious clinical symptoms. 90% of affected children are detected within two years of age, and the incidence is essentially similar in boys and girls. Its pathogenesis is mainly due to a congenital malformation of the lymphoid tissue, where the junction of the internal jugular and subclavian veins is enlarged during embryonic life, forming a cystic cavity called the cervical sac. If a portion of the lymphatic system develops from the cervical sac, during embryonic development, if a portion of the lymphatic tissue becomes vagal and remains embryonic in nature, it continues to develop or grows in size and becomes a multi-housed cystic mass containing lymphatic fluid or covered with endothelium, forming a cystic hydatid tumor.